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Mayor Erythropoietic Response after Deferasirox Treatment in a Transfusion-Dependent Anemic Patient with Primary Myelofibrosis

DOI: 10.1155/2013/520712

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Abstract:

Primary myelofibrosis (PMF) is a myeloproliferative neoplasm frequently complicated by transfusion dependent anemia. Both anemia and transfusion-dependence are associated with a poor outcome, at least in part because of toxic effects of iron overload (IOL). Iron-chelating therapy (ICT) is increasingly used in order to prevent IOL in this setting. Here, we describe the case of a 73-year-old man affected by PMF and severe transfusion-dependent anemia who experienced a dramatic erythroid response after being treated with deferasirox to prevent IOL. 1. Introduction Anemia is a frequent complication of primary myelofibrosis (PMF), either at presentation or during the course of the disease, with an incidence and diagnosis ranging from 50 to 70% [1]. The presence of anemia in PMF is well known to negatively impact survival, and transfusion dependence has been recently reported as a further negative prognostic factor; both of these variables are included in the more used current PMF prognostic scores [2, 3]. Although severe anemia could per se indicate a more aggressive disease with higher risk of leukemic transformation, the increased mortality in severely anemic PMF patients does not seem to be related entirely to leukemia but also to the negative effects of chronic low hemoglobin levels on cardiovascular system, and, in the heavily transfused patients, it might also be dependent on the systemic damage of the heart and other organs due to iron overload (IOL). IOL is also believed to increase the infective risk of these already frail patients. To limit the toxicity of iron excess, iron-chelating therapy (ICT), although not routinely recommended by current guidelines of PMF management, has been recently increasingly proposed in the management of these patients, when transfusion-dependent anemia occurs. A positive effect from ICT on survival in patients with PMF has been already demonstrated by Leitch et al. [4], and it was mainly attributed to a reduction of toxic effects of IOL. A possible direct effect of ICT in improving erythropoiesis of patients with PMF has also been described, even if in a few cases [5–8]. Here, we describe a PMF patient with severe transfusion-dependent anemia in which ICT with deferasirox stunningly restored normal hemoglobin levels. 2. Case Presentation A 73-year-old Caucasian otherwise healthy man came to our outpatient’s clinic in August 2011 because of neutrophil leukocytosis and splenomegaly. Blood counts were as follows: white blood cells (WBC) 28.2 × 109/L, hemoglobin (Hb) 11.5?g/dL, and platelets (Plt) 350 × 109/L. Physical

References

[1]  G. Barosi, “Myelofibrosis with myeloid metaplasia: diagnostic definition and prognostic classification for clinical studies and treatment guidelines,” Journal of Clinical Oncology, vol. 17, no. 9, pp. 2954–2970, 1999.
[2]  B. Dupriez, P. Morel, J. L. Demory et al., “Prognostic factors in agnogenic myeloid metaplasia: a report on 195 cases with a new scoring system,” Blood, vol. 88, no. 3, pp. 1013–1018, 1996.
[3]  A. Tefferi, “Primary myelofibrosis: 2013 update on diagnosis, risk-stratification, and management,” American Journal of Hematology, no. 2, pp. 141–150, 2013.
[4]  H. A. Leitch, J. M. Chase, T. A. Goodman et al., “Improved survival in red blood cell transfusion dependent patients with primary myelofibrosis (PMF) receiving iron chelation therapy,” Hematological Oncology, vol. 28, no. 1, pp. 40–48, 2010.
[5]  J. H. Marsh, M. Hundert, and P. Schulman, “Deferoxamine-induced restoration of haematopoiesis in myelofibrosis secondary to myelodysplasia,” British Journal of Haematology, vol. 76, no. 1, pp. 148–149, 1990.
[6]  M. E. Smeets, G. Vreugdenhil, and R. S. Holdrinet, “Improvement of erythropoiesis during treatment with deferiprone in a patient with myelofibrosis and trasfusional hemosiderosis,” American Journal of Hematology, vol. 51, no. 3, pp. 243–244, 1996.
[7]  E. Messa, D. Cilloni, F. Messa, F. Arruga, A. Roetto, and G. Saglio, “Deferasirox treatment improved the hemoglobin level and decreased transfusion requirements in four patients with the myelodysplastic syndrome and primary myelofibrosis,” Acta Haematologica, vol. 120, no. 2, pp. 70–74, 2008.
[8]  A. A. Di Tucci, R. Murru, D. Alberti, B. Rabault, S. Deplano, and E. Angelucci, “Correction of anemia in a transfusion-dependent patient with primary myelofibrosis receiving iron chelation therapy with deferasirox (Exjade ICL670),” European Journal of Haematology, vol. 78, no. 6, pp. 540–542, 2007.
[9]  A. Tefferi, J. Thiele, and J. W. Vardiman, “The 2008 world health organization classification system for myeloproliferative neoplasms: order out of chaos,” Cancer, vol. 115, no. 17, pp. 3842–3847, 2009.
[10]  F. Cervantes, B. Dupriez, A. Pereira et al., “New prognostic scoring system for primary myelofibrosis based on a study of the International working group for myelofibrosis research and treatment,” Blood, vol. 113, no. 13, pp. 2895–2901, 2009.
[11]  F. Passamonti, F. Cervantes, A. M. Vannucchi et al., “A dynamic prognostic model to predict survival in primary myelofibrosis: a study by the IWG-MRT (International Working Group for Myeloproliferative Neoplasms Research and Treatment),” Blood, vol. 115, no. 9, pp. 1703–1708, 2010.
[12]  A. Tefferi, F. Cervantes, R. Mesa, et al., “Revised response criteria for myelofibrosis: international Working Group-Myeloproliferative Neoplasms Research and Treatment (IWG-MRT) and European LeukemiaNet (ELN) consensus report,” Blood, vol. 122, pp. 1395–1398, 2013.
[13]  A. Tefferi, R. A. Mesa, A. Pardanani et al., “Red blood cell transfusion need at diagnosis adversely affects survival in primary myelofibrosis—increased serum ferritin or transfusion load does not,” American Journal of Hematology, vol. 84, no. 5, pp. 265–267, 2009.
[14]  A. Pardanani, C. Finke, R. A. Abdelrahman, T. L. Lasho, and A. Tefferi, “Associations and prognostic interactions between circulating levels of hepcidin, ferritin and inflammatory cytokines in primary myelofibrosis,” American Journal of Hematology, vol. 88, pp. 312–316, 2013.
[15]  R. A. Mesa, M. A. Elliott, and A. Tefferi, “Splenectomy in chronic myeloid leukemia and myelofibrosis with myeloid metaplasia,” Blood Reviews, vol. 14, no. 3, pp. 121–129, 2000.
[16]  C. Rose, S. Brechignac, D. Vassilief et al., “Does iron chelation therapy improve survival in regularly transfused lower risk MDS patients? A multicenter study by the GFM,” Leukemia Research, vol. 34, no. 7, pp. 864–870, 2010.
[17]  H. Gattermann, C. Finelli, M. D. Porta, et al., “Hematologic responses to deferasirox therapy in trasfusion-dependent patients with myelodysplatic syndromes,” Haematologica, vol. 97, no. 9, pp. 1364–1371, 2012.
[18]  R. Guariglia, M. C. Martorelli, O. Villani et al., “Positive effects on hematopoiesis in patients with myelodysplastic syndrome receiving deferasirox as oral iron chelation therapy: a brief review,” Leukemia Research, vol. 35, no. 5, pp. 566–570, 2011.
[19]  P. D. Jensen, L. Heickendorff, B. Pedersen et al., “The effect of iron chelation on haemopoiesis in MDS patients with transfusional iron overload,” British Journal of Haematology, vol. 94, no. 2, pp. 288–299, 1996.
[20]  H. Ghoti, J. Amer, A. Winder, E. Rachmilewitz, and E. Fibach, “Oxidative stress in red blood cells, platelets and polymorphonuclear leukocytes from patients with myelodysplastic syndrome,” European Journal of Haematology, vol. 79, no. 6, pp. 463–467, 2007.
[21]  L. Chan, R. Buckstein, M. Reis et al., “P092 Iron overload and haematopoiesis in MDS: does blood transfusion promote progression to AML?” Leukemia Research, vol. 33, no. 1, pp. S112–S113, 2009.
[22]  E. Messa, S. Carturan, C. Maffè et al., “Deferasirox is a powerful NF-κb inhibitor in myelodysplastic cells and in leukemia cell lines acting independently from cell iron deprivation by chelation and reactive oxygen species scavenging,” Haematologica, vol. 95, no. 8, pp. 1308–1316, 2010.

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